Clinical Guidelines for Management of Acute Painful Episodes of Vaso-Occlusive Crisis in Children with Sickle Cell Disease

Nawaf Alanazi


Sickle cell disease (SCD) is an inherited disease resulted due to a single mutation in beta globin gene. It changes normal red blood cells into sickle-shaped cells, just rendering their ability to carry oxygen to tissues and cells. SCD involves many fatal clinical complications including very painful vaso-occlusive crisis (VOC), the later caused by sickled erythrocytes-mediated ischemic tissue injury and obstruction of blood flow that leads. Pain in VOC is caused by hypoxia, acidosis, fever, infections, dehydration, obstructive sleep apnea, abrupt changes in weather (hot / cold), menstruation and pregnancy etc. Pain in VOC is very severe and is associated with many other life-threatening clinical complications. Therefore, it is needed be managed as early as possible, otherwise persistent pain can weaken patients physically and psychologically. There are no specific laboratory indicators for VOC and diagnosis is made on the basis of history and physical examination. We formulated clinical guidelines for the management of acute painful crisis (VOC) in sickle cell patients based upon updated published medical literature, in addition to our experience of treating sickle cell patients King Abdulaziz National Guards Hospital, Al-Ahsa, Saudi Arabia, which is its application to an Asian publication. This report describes details of these clinical guidelines for management of acute painful vaso-occlusive crisis in sickle cell patients.

Keywords: Sickle cell disease; Vaso-Occlusive Crisis; Acute pain management; Pediatric

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